What to Know About the Treatment of Childhood Soft Tissue Sarcoma

The treatment for childhood soft tissue sarcoma depends on the specific type and extent of the cancer. It often involves surgery to remove the cancer and potentially involves chemotherapy or radiation therapy.

Soft tissue sarcoma (STS) is a type of cancer that develops in the soft tissues of the body, including but not limited to the muscles, fat, and blood vessels. This type of cancer can form in any part of the body.

While STS can occur at any age, it’s more common in children. This article will explore the treatments that may be used for childhood STS as well as their potential risks and side effects.

The goal of treatment for childhood STS depends on the extent of the cancer at diagnosis. This is called the stage of the cancer.

If the cancer is found while it’s still in an early stage, it may be possible to remove it completely. In this case, the goal would be to cure the cancer.

Almost two-thirds of that $3.3 trillion cost – 64% – is paid for by American tax dollars, and that amount is growing. A study by the American Journal of Public Health predicts that taxpayers will shoulder 67.3% of the burden of healthcare costs by the year 2024, Physicians for a National Health Program

However, if the cancer is more advanced, the goal of treatment may be aimed more at managing symptoms and preventing the cancer from progressing further.

It’s important to note that STS, in general, is very diverse. Because of this, treatment approaches will vary based on the specific type and extent of your child’s cancer.

Surgery is a very prominent part of treating childhood STS. According to the American Cancer Society , surgical removal provides the best chance of curing STS.

Whether or not a tumor can be safely removed using surgery depends on several factors. These include:

• the size of the tumor
• where the tumor is located
• if the tumor is close to any vital organs or tissues

Generally speaking, tumor removal involves a procedure called wide local excision. This removes the cancer as well as a little bit of the healthy tissue around it.

In very rare cases, amputation of part of all of a limb may be done in order to completely remove the cancer.

Side effects and risks of surgery

The specific side effects and risks related to surgery for childhood STS can vary based on the site of the surgery. However, some general side effects and risks are:

• a bad reaction to the anesthetics used
• bleeding
• infections after surgery
• serious blood clots
• damage to surrounding organs or tissues
• long-term effects on function, depending on the area involved

Chemotherapy (chemo) uses drugs that affect the growth and division of rapidly dividing cells like cancer cells. There are a few scenarios in which chemo is used for childhood STS:

• before surgery to shrink the size of the tumor (neoadjuvant therapy)
• after surgery to help kill any remaining cancer cells (adjuvant therapy)
• as a main treatment when the cancer can’t be treated with surgery

The type of chemo drugs that are used depends on the exact type of STS. Some types of childhood STS are more responsive to chemo than others. Examples of chemo drugs that may be used for childhood STS are:

• vincristine
• dactinomycin
• cyclophosphamide
• doxorubicin
• ifosfamide
• docetaxel
• gemcitabine
• trabectedin

Side effects and risks of chemotherapy

Because chemo targets rapidly dividing cells, it can also affect healthy cells that grow and divide quickly. These include cells in the bone marrow, hair follicles, and digestive tract. The short-term side effects of chemo are:

• low blood cell counts, which can lead to:
  • anemia
  • frequent infections
  • easy bruising or bleeding
  • reduced appetite
  • mouth sores
  • nausea and vomiting
  • constipation
  • diarrhea

  There are also long-term effects of chemo. This is particularly important in children since they’re still growing, meaning many cell types are growing and dividing more rapidly. Examples of potential long-term effects of chemo are:

  • a slowdown in growth
  • problems with certain organs and tissues, including but not limited to the heart, teeth, and digestive tract
  • future issues with fertility
  • increased risk of second cancers

  Radiation therapy uses high-energy radiation to kill cancer cells. There are a few ways radiation therapy may be used for childhood STS:

  • as a neoadjuvant therapy
  • as an adjuvant therapy
  • as a main treatment when the cancer can’t be treated with surgery
  • to help ease symptoms when cancer has spread

  Many times, radiation therapy is delivered from a machine outside of the body. This is called external beam radiation.

  In some scenarios, brachytherapy may be used. This involves placing a catheter, needle, or another implant that contains radioactive material into the body near the cancer.

  Sometimes chemo drugs help radiation therapy to work better. As such, radiation therapy may also be used along with chemo. This is called chemoradiation.

  Side effects and risks of radiation therapy

  The exact side effects of radiation therapy can depend on the location of the body where it’s given as well as the dose of radiation that’s used. Some of the potential side effects and risks include:

  • fatigue
  • digestive symptoms like nausea and vomiting and diarrhea
  • skin changes, such as redness, blistering, or peeling
  • hair loss
  • headache
  • throat and mouth problems, such as trouble swallowing
  • shortness of breath
  • urinary or bladder problems
  • trouble with memory and concentration
  • future issues with fertility
  • increased risk of second cancers

  Targeted therapy uses drugs that are designed to target specific markers found on or in cancer cells. They’re often used for cancer that’s more advanced or that has metastasized.

  Targeted therapy drugs are typically given for childhood STS as a part of a clinical trial.

  Examples of targeted therapy drugs that are being investigated for childhood STS are:

  • crizotinib (Xalkori)
  • imatinib (Gleevec)
  • larotrectinib (Vitrakvi)
  • pazopanib (Votrient)
  • sorafenib (Nexavar)
  • sunitinib (Sutent)
  • temsirolimus (Torisel)

  Side effects and risks of targeted therapy

  While targeted therapy is often less harmful to healthy cells than chemo or radiation therapy, it still has some potential side effects. These may vary widely based on the specific targeted therapy drug used.

  Some general side effects may include:

  • fatigue
  • digestive symptoms like:
    • reduced appetite
    • nausea and vomiting
    • constipation
    • diarrhea

    Immunotherapy helps the immune system to respond to cancer. Like targeted therapy drugs, immunotherapy may be used for more advanced or metastatic STS.

    Immunotherapies are currently being approved for children with STS based on the results of promising clinical trials. These recently approved immunotherapy drugs include:

    • pembrolizumab (Keytruda)
    • atezolizumab

    Side effects and risks of immunotherapy

    The potential side effects and risks of immunotherapy can include:

    • a reaction while receiving the drug, called an infusion reaction
    • fatigue
    • digestive symptoms like:
      • reduced appetite
      • nausea and vomiting
      • constipation
      • diarrhea

      If your child has received a diagnosis of STS, you may have many questions swirling in your head. It’s important to raise these questions to your child’s care team so that you can get as much information as possible about their treatment.

      Some examples of questions to ask include:

      • What type of STS does my child have? What stage is it?
      • What kind of treatment do you recommend for my child’s STS?
      • Is surgery an option for my child? If so, what will it involve? Will other types of treatment be used as well?
      • If my child isn’t eligible for surgery, what kinds of treatment will be used?
      • How will my child’s treatment affect their outlook?
      • When will my child need to start treatment?
      • Where will my child receive treatment and how often?
      • What are the potential side effects of my child’s treatment? How can I help to manage or prevent them?
      • Are there any long-term side effects associated with this treatment? If so, what are they?
      • Will health insurance cover the cost of my child’s treatment? If not, what can I do?

      Aim to write your questions down and bring them to your child’s appointment. It’s recommended to leave some space under each question so you can take notes to reference later.

      How common is childhood soft tissue sarcoma?

      While STS is rare in adults, it’s more common in children. In fact, STS makes up about 8% of all cancers in children and adolescents. About half of childhood STS diagnoses are rhabdomyosarcomas.

      Who gets childhood soft tissue sarcoma?

      Some factors can put a child at a higher risk of STS. These include having inherited disorders, such as Li-Fraumeni syndrome and familial adenomatous polyposis, as well as having previous exposure to radiation therapy.

      How serious is childhood soft tissue sarcoma?

      Some types of childhood STS are more aggressive than others. As such, it’s best to discuss your child’s individual outlook with their care team.